What is Pulmonary Fibrosis?
Pulmonary fibrosis is a disease that scars the lungs, causing intricate pulmonary passageways to thicken and harden and obstructing the free passage of oxygen through the walls of the lungs’ tiny air sacs (alveoli) into the bloodstream. The resulting lack of oxygen in the bloodstream leaves pulmonary fibrosis sufferers short of breath even after periods of prolonged rest. Idiopathic pulmonary fibrosis is a form of pulmonary fibrosis with no known cause. Pulmonary fibrosis is a progressive disease that varies in the rate of degeneration from person to person.
Pulmonary fibrosis is a subset of a group of conditions referred to as interstitial lung disease.
The term interstitial lung disease refers to conditions that lead to inflammation or scarring of the lung’s delicate tissues. Some autoimmune disorders, specifically connective tissue disorders like rheumatoid arthritis can cause interstitial lung disease. Scarring of the lungs is irreversible. However, treatment options are available.
There are many causes of pulmonary fibrosis including occupational and environmental factors, reaction to medications, autoimmune disorders, infection, genetics and unknown factors. Smoking exacerbates the condition and may lead to cancer and/or other lung conditions.
Occupational and Environmental Factors:
Exposure to toxins or other pollutants for a long period of time can cause major damage to the lungs.
- Silica dust
- Asbestos fibers
- Grain dust
- Bird and animal feces
- Burn Pits
Individuals who have received radiation therapy or have used certain medications for an extended period may be more susceptible.
- Radiation therapy
- Chemotherapeutic agents
The role of genetics in the onset of pulmonary fibrosis is still being evaluated. It is estimated that 10 to 15 percent of patients with idiopathic pulmonary fibrosis have some form of inherited pulmonary fibrosis, referred to as familial pulmonary fibrosis.
- Familial pulmonary fibrosis
- Hermansky-Pudlak syndrome
Sufferers of autoimmune diseases–specifically connective tissue disorders–can develop pulmonary fibrosis. These autoimmune disorders can lead to pulmonary fibrosis:
- Scleroderma or progressive systemic sclerosis
- Rheumatoid arthritis
- Polymyositis or dermatomyositis
Severe infections can contribute to multiple types of interstitial lung diseases, including pulmonary fibrosis.
- Viral infections
- Bacterial infections
The symptoms of pulmonary fibrosis differ by individual.
Sometimes symptoms progress very quickly. In other cases, symptoms remain mild to moderate for years, until progressing rapidly in later stages. Sufferers may experience episodes in which their symptoms suddenly worsen; these episodes are referred to as acute flare-ups of the disease. Shortness of breath is the most common complaint of pulmonary fibrosis sufferers, other symptoms include:
- Achy joints
- Weight loss
Typically, sufferers of pulmonary fibrosis do not live more than 5 years after a diagnosis.
Idiopathic pulmonary fibrosis has no specific demographic. However it does affect men more than women. It typically occurs in individuals between the age of 50 and 70, and has an increased risk for individuals who smoke cigarettes.
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